Honsová Eva
Cesk Patol. 2020 Spring;56(2):68-73.
The most common group of systemic vasculitides in adulthood are anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). AAV represent autoimmune systemic vasculitides and include 3 clinical phenotypes: Granulomatosis with polyangiitis (GPA, formerly Wegener granulomatosis), Microscopic polyangiitis (MPA) and Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome). Histological features are similar to each other in all affected locations, and there are represented by necrotizing vascular inflammation of small and medium calibers, often venules, capillaries or arteriols, typically with fibrinoid vessel wall necrosis. The consequences of this condition are bleeding, as well as compromise of the lumen which may result in downstream tissue ischemia and necrosis. Typically affected locations in biopsy practice are: ENT, lung, skin, GIT, and kidney. The aim of this review is to provide a comprehensive overview of the important histopathological findings. ANCA positive vasculitis is a serious life-threatening disease and therefore requires a rapid diagnosis and appropriate therapy.
成人中最常见的一类系统性血管炎是抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）。AAV是自身免疫性系统性血管炎，包括3种临床表型：肉芽肿性多血管炎（GPA，原韦格纳肉芽肿）、显微镜下多血管炎（MPA）和嗜酸性肉芽肿性多血管炎（EGPA，原变应性肉芽肿性血管炎）。所有受累部位的组织学特征彼此相似，表现为中小口径血管的坏死性血管炎症，通常是小静脉、毛细血管或小动脉，典型表现为血管壁纤维素样坏死。这种情况的后果是出血，以及管腔受压，这可能导致下游组织缺血和坏死。活检实践中典型的受累部位是：耳鼻喉、肺、皮肤、胃肠道和肾脏。本综述的目的是全面概述重要的组织病理学发现。ANCA阳性血管炎是一种严重的危及生命的疾病，因此需要快速诊断和适当治疗。
